• 1
    Bone RC. Gram-negative sepsis – a dilemma of modern medicine. Clin Microbiol Rev 1993; 6:5768.
  • 2
    Brandtzaeg P. Significance and pathogenesis of septic shock. Curr Top Microbiol 1996; 216:1537.
  • 3
    Morrison DC, Danner RL, Dinarello CA et al. Bacterial endotoxins and pathogenesis of Gram-negative infections: current status and future direction. J Endotoxin Res 1994; 1:7183.
  • 4
    Brandtzaeg P, Bryn K, Kierulf P, Øvstebø R, Namork E, Aase B, Jantzen E. Meningococcal endotoxin in lethal septic shock plasma studied by gas chromatography, mass-spectrometry, ultracentrifugation, and electron microscopy. J Clin Invest 1992; 89:81623.
  • 5
    Brandtzaeg P, Kierulf P, Gaustad P, Skulberg A, Bruun JN, Halvorsen S, Sørensen E. Plasma endotoxin as a predictor of multiple organ failure and death in systemic meningococcal disease. J Infect Dis 1989; 159:195204.
  • 6
    Moran AP. Structure-bioactivity relationships of bacterial endotoxins. J Toxicol Toxin Rev 1995; 14:4783.
  • 7
    Rietschel ET, Brade H, Holst O et al. Bacterial endotoxin: chemical constitution, biological recognition, host response, and immunological detoxification. Curr Top Microbiol 1996; 216:3981.
  • 8
    Hsueh W, Sun X, Rioja LN, Gonzalez-Crussi F. The role of the complement system in shock and tissue injury induced by tumor necrosis factor and endotoxin. Immunology 1990; 70:30914.
  • 9
    Brandtzaeg P, Høgåsen K, Kierulf P, Mollnes TE. The excessive complement activation in fulminant meningococcal septicemia is predominantly caused by alternative pathway activation. J Infect Dis 1996; 173:64755.
  • 10
    Karima R, Matsumoto S, Higashi H, Matsushima K. The molecular pathogenesis of endotoxic shock and organ failure. Mol Med Today 1999; 5:12332.DOI: 10.1016/s1357-4310(98)01430-0
  • 11
    Müller-Eberhard HJ. The membrane attack complex of complement. Annu Rev Immunol 1986; 4:50328.
  • 12
    Harriman GR, Esser AF, Podack ER, Wunderlich AC, Braude AI, Lint TF, Curd JG. The role of C9 in complement-mediated killing of Neisseria. J Immunol 1981; 127:238690.
  • 13
    Pramoonjago P, Kinoshita T, Hong K, Takata-Kozono Y, Kozono H, Inagi R, Inoue K. Bactericidal activity of C9-deficient human serum. J Immunol 1992; 148:83743.
  • 14
    Joiner KA, Schmetz MA, Sanders ME, Murray TG, Hammer CH, Dourmaskin R, Frank MM. Multimeric complement component C9 is necessary for killing of Escherichia coli J5 by terminal attack complex C5b-9. Proc Natl Acad Sci USA 1985; 82:480812.
  • 15
    MacKay SLD & Dankert JR. Bacterial killing and inhibition of inner membrane activity by C5b-9 complexes as a function of the sequential addition of C9 to C5b-8 sites. J Immunol 1990; 145:336771.
  • 16
    Bloch EF, Knight EM, Carmon T et al. C5b-7 and C5b-8 precursors of the membrane attack complex (C5b-9) are effective killers of E. coli J5 during serum incubation. Immunol Invest 1997; 26:40919.
  • 17
    Wang Y, Bjes ES, Esser AF. Molecular aspects of complement-mediated bacterial killing. Periplasmic conversion of C9 from a protoxin to a toxin. J Biol Chem 2000; 275:468792.
  • 18
    Fukumori Y, Yoshimura K, Ohnoki S, Yamaguchi H, Akagaki Y, Inai S. A high incidence of C9 deficiency among healthy blood donors in Osaka, Japan. Int Immunol 1989; 1:859.
  • 19
    Nagata M, Hara T, Aoki T, Mizuno Y, Akeda H, Inaba S, Tsumoto K, Ueda K. Inherited deficiency of ninth component of complement: an increased risk of meningococcal meningitis. J Pediatr 1989; 114:2604.
  • 20
    Würzner R, Orren A, Lachmann PJ. Inherited deficiencies of the terminal components of human complement. Immunodefic Rev 1992; 3:12347.
  • 21
    Tesh VL, Duncan RL, Morrison DC. The interaction of Escherichia coli with normal human serum: the kinetics of serum-mediated lipopolysaccharide release and its dissociation from bacterial killing. J Immunol 1986; 137:132935.
  • 22
    Lehner PJ, Davies KA, Walport MJ, Cope AP, Würzner R, Orren A, Morgan BP, Cohen J. Meningococcal septicaemia in a C6-deficient patient and effects of plasma transfusion on lipopolysaccharide release. Lancet 1992; 340:137981.
  • 23
    Fischer MB, Prodeus AP, Nicholson-Weller A et al. Increased susceptibility to endotoxin shock in complement C3- and C4- deficient mice is corrected by C1 inhibitor replacement. J Immunol 1997; 159:97682.
  • 24
    Rietschel ET & Brade H. Bacterial endotoxins. Sci Am 1992; 267:5461.
  • 25
    Elbein AD & Heath EC. The biosynthesis of cell wall lipopolysaccharide in Escherichia coli. I. The biochemical properties of a uridine diphosphate galactose-4-epimerlaseless mutant. J Biol Chem 1965; 240:191925.
  • 26
    Edstrom RD & Heath EC. The biosynthesis of cell wall lipopolysaccharide in Escherichia coli. VII. Studies on the structure of the O-antigenic polysaccharide. J Biol Chem 1967; 242:412533.
  • 27
    Appelmelk BJ, Maaskant JJ, Verweij-van Vught AMJJ, Van der Meer NM, Thijs BG, MacLaren DM. Antigenic and immunogenic differences in lipopolysaccharides of Escherichia coli J5 vaccine strains of different origins. J Gen Microbiol 1993; 139:26417.
  • 28
    Müller-Loennies S, Holst O, Brade H. Chemical structure of the core region of Escherichia coli J5 lipopolysaccharide. Eur J Biochem 1994; 224:75160.
  • 29
    Müller-Loennies S, Holst O, Lindner B, Brade H. Isolation and structural analysis of phosphorylated oliogsaccharides obtained from Escherichia coli J5 lipopolysaccharide. Eur J Biochem 1999; 260:23549.DOI: 10.1046/j.1432-1327.1999.00134.x
  • 30
    Orren A, Potter PC, Cooper RC, Du Toit E. Deficiency of the sixth component of complement and susceptibility to Neisseria meningitidis infections: studies in 10 families and five isolated cases. Immunology 1987; 62:24953.
  • 31
    O'Hara AM, Fernie BA, Moran AP, Williams YE, Connaughton JJ, Orren A, Hobart MJ. C7 deficiency in an Irish family: a deletion defect which is predominant in the Irish. Clin Exp Immunol 1998; 114:33561.
  • 32
    O'Toole G, Seifer CM, Crowley DC, Sheehan GJ, Orren A. The diagnosis of complement deficiency: studies of two Irish families with recurrent meningococcal disease (abstr.). Irish J Med Sci 1995; 164:910.
  • 33
    Hobart MJ, Fernie BA, Würzner R, Oldroyd RG, Harrison RA, Joysey V, Lachmann PJ. Difficulties in the ascertainment of C9 deficiency: lessons to be drawn from a compound heterozygote C9-deficient subject. Clin Exp Immunol 1997; 108:5006.
  • 34
    Witzel-Schlömp K, Hobart MJ, Fernie BA, Orren A, Würzner R, Rittner C, Kaufmann T, Schneider PM. Heterogeneity in the genetic basis of human complement C9 deficiency. Immunogenetics 1998; 48:1447.DOI: 10.1007/s002510050415
  • 35
    Leive L. Actinomycin sensitivity in Escherichia coli produced by EDTA. Biochem Biophys Res Commun 1965; 18:137.
  • 36
    Duncan RL & Morrison DC. The fate of E. coli lipopolysaccharide after the uptake of E. coli by murine macrophages in vitro. J Immunol 1984; 132:141624.
  • 37
    Galanos G & Lüderitz O. Electrodialysis of lipopolysaccharides and their conversion to uniform salt forms. Eur J Biochem 1975; 54:60310.
  • 38
    Morgan BP, Daw RA, Siddle K, Luzio JP, Campbell AK. Immunoaffinity purification of human complement C9 using monoclonal antibodies. J Immunol Methods 1983; 64:26981.
  • 39
    Würzner R, Orren A, Potter P et al. Functionally active complement proteins C6 and C7 detected in C6- and C7-deficient individuals. Clin Exp Immunol 1991; 83:4307.
  • 40
    Rietschel ET, Kirikae T, Schade FU et al. Bacterial endotoxin: molecular relationships of structure to activity and function. FASEB J 1994; 8:21725.
  • 41
    Brandenburg K, Mayer H, Koch MHJ, Weckesser J, Rietschel ET, Seydel U. Influence of the supramolecular structure of free lipid A on its biological activity. Eur J Biochem 1993; 218:55563.
  • 42
    Takayama K, Mitchell DH, Din ZZ, Mukerjee P, Li C, Coleman DL. Monomeric Re lipopolysaccharide from Escherichia coli is more active than the aggregated form in the Limulus amebocyte lysate assay and in inducing Egr-1 mRNA in murine peritoneal macrophages. J Biol Chem 1994; 269:22414.
  • 43
    Kitchens RL & Munford RS. CD14-dependent internalization of bacterial lipopolysaccharide (LPS) is strongly influenced by LPS aggregation but not by cellular responses to LPS. J Immunol 1998; 160:19208.
  • 44
    Tesh VL & Morrison DC. The interaction of Escherichia coli with normal human serum: factors affecting the capacity of serum to mediate lipopolysaccharide release. Microb Pathog 1988; 4:17587.
  • 45
    Würzner R, Platonov AE, Beloborodov VB et al. How partial C7 deficiency with chronic and recurrent bacterial infections can mimic total C7 deficiency: temporary restoration of host C7 levels following plasma infusion. Immunology 1996; 88:40711.
  • 46
    Orren A, Würzner R, Potter PC, Fernie BA, Coetzee S, Morgan BP, Lachmann PJ. Properties of a low molecular weight complement component C6 found in human subjects with subtotal C6 deficiency. Immunology 1992; 75:106.
  • 47
    Esser AF. The membrane attack complex of complement. Assembly, structure and cytotoxic activity. Toxicology 1994; 87:22947.